Mannose 6-phosphate receptors, Niemann-Pick C2 protein, and lysosomal cholesterol accumulation

Mannose 6-phosphate receptors, Niemann-Pick C2 protein, and lysosomal cholesterol accumulation

Niemann-Pick disease type C (NPC), caused by mutations in the NPC1 gene or the NPC2 gene, is characterized by the accumulation of unesterified cholesterol and other lipids in endo/lysosomal compartments. NPC2 is a small, soluble, lysosomal protein that is targeted to this compartment via a mannose 6...

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Bibliographic Details
Main Authors: Marion Willenborg, Christine Kathrin Schmidt, Peter Braun, Jobst Landgrebe, Kurt von Figura, Paul Saftig, Eeva-Liisa Eskelinen
Format: Article
Language:English
Published: Elsevier 2005-12-01
Series:Journal of Lipid Research
Subjects:
NPC1
NPC2
cholesterol
late endosome
Online Access:http://www.sciencedirect.com/science/article/pii/S0022227520328431

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http://www.sciencedirect.com/science/article/pii/S0022227520328431

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